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Pulmonary Manifestations of
LUPUS
Mohmeet Singh Brar
PG Resident
Introduction
SLE( systemic lupus erythematosus)
• Autoimmune
• Microvascular inflammation
• 10:1 female to male ratio
• Antibodies directed against double stranded
DNA,1 nuclear ribonucleoprotein, Smith (Sm)
antigen, Ro/SS-A, and La/SS-B/Ha.
Pleuropulmonary manifestations
• Pleuritis with or without effusion
• Upper and lower airway infections
• Acute Lupus Pneumonitis
• Chronic interstitial pneumonia
• Organizing pneumonia
• Pulmonary hypertension
• Pulmonary embolism
• Respiratory muscle weakness (shrinking lung syndrome)
• Alveolar hemorrhage
• Mediastinal lymphadenopathy
Allergy 2005; 60: 715-734
Pleural involvement
• Most common manifestation
• Asymptomatic
• Musculoskeletal pain
• Pleuritic chest pain (45-60 %)
• Effusions (50-70%)
• Dyspnea
• Fever
• Pleural effusion: B/L
exudative, lymphocytic predominant
high glucose, low LDH, complement levels
LE cells specific
• Treatment:
Minimal: no treatment, conservative
Pleuritic pain: NSAIDs
Severe Ds: corticosteroids
Chest tube drain/pleurodesis: rarely req
CXR showing left sided pleural effusion.
CT Chest showing B/L pleural effusion
UPPER RESPIRATORY TRACT
INFECTION
• Cough : mc symptom
• Viral
• Patients taking corticosteroids or
immunosuppresives
• Treatment: symptomatic
cough syrups,
steam inhalation
Acute lupus pneumonitis
• Non specific
• May simulate infections
• 1-4 % of patients
• Cough, dyspnoea, pleuritic
pain, hypoxaemia, and fever
• CXR: diffuse acinar infiltrates( u/l as well as
b/l)
• Sterile sputum/ ET cultures
• Histologicaly:
alveolar wall damage and necrosis,
inflammatory cell infiltration,
oedema, haemorrhage,hyaline membrane
• CT Scan:
alveolitis (a ground glass appearance)
fibrosis (a honey comb appearance)
• BAL: lymphocytic predominance
sterile cultures
• Gallium scintigraphy: increased uptake
• DLCO : decreased
• Video-assisted thoracoscopic biopsy or open
lung biopsy: last option
Diffuse acinar infiltrates in the right lower zone
ARROW showing basal cosolidation with atelectasis
Treatment:
• Broad spectrum antibiotic cover
• Systemic prednisone (1 to 1.5 mg/kg per day
in divided doses)
• Intravenous pulse glucocorticoids (1 gram of
methylprednisolone/ day for 3days)
immunosuppressive drugs
(cyclophosphamide)
Sick patients( tachypnea, hypoxemia)
Patients with no response after 72 hours of
prednisone
• PROGNOSIS:
Fulminant course
High mortality
Poor prog: BAL with eosinophils, postpartum
CXR showing right lower zone
shadows
Improvement of CXR after 4
weeks of therapy
INTERSTITIAL LUNG DISEASE
• Upto 9 %
• Clinical features:
Chronic nonproductive cough
Dyspnea
Decreased exercise tolerance
• Diagnosis: pulmonary function tests with
exprapulmonary lupus
• PFT: restrictive pattern
decreased TLC
decreased DLCO
• HRCT CHEST: ground glass appearance
centrilobular nodules
thickened bronchovascular
bundles and airspaces
• LUNG BIOPSY: cellular infiltration with fibrotic
changes
Patterns include
• Nonspecific interstitial pneumonia (NSIP),
• Usual interstitial pneumonia(UIP),
• Lymphocytic interstitial pneumonia (LIP),
• Cryptogenic organizing pneumonia
Chronic interstitial pneumonia in a 35 year old woman
with SLE.HRCT scan shows extensive ground glass
opacities admixed with coarse linear bands and
honeycomb cysts.
• TREATMENT: depends upon inflammatory or
fibrotic pattern
• Dual therapy
• High glucocorticoids (prednisone 1 to 2
mg/kg/day) and cyclophosphamide
• Transition to either azathioprine or
mycophenolate mofetil after 6 to 12 months.
• Nothing established for fibrotic disease
• PIRFENIDONE( tyrosine kinase inhibitor): trial
basis
PULMONARY HYPERTENSION
• Rare complication
• 5-10% of patients
• Increases with age
• 2 % have right heart failure
• Dyspnea
• Chest pain
• Chronic non productive cough
• Prominent JVP
• Hepatomegaly
• Ascites
• Peripheral edema
• CXR: enlarged pulmonary arteries with clear
lung fields.
straightening of the left heart border and
attenuation of the peripheral vessels
CXR showing straightening of the left heart border and
attenuation of the peripheral vessels
• ECG: RVH
• PFT: restrictive pattern
decreased DLCO
• 2 D ECHO: inc PAP and TR ( tricuspid valve
insufficiency)
• CT CHEST: dilatation of main pulmonary artery
and heterogenesity of lung perfusion
• TREATMENT:
oxygen
anticoagulants
vasodilators :bosentan,
calcium channel blockers,
prostacyclin,
endothelial antagonists,
sildenafil
Intermittent Cyclophosphamide pulse
Lupus 2004; 13:105
SHRINKING LUNG SYNDROME
• Less common manifestation
• Dyspnea,
• Pleuritic chest pain
• Progressive decrease in lung volume
• No evidence of interstitial fibrosis or pleural
disease on chest CT
• myositis or myopathy affecting both
diaphragms
• DIAGNOSIS: triad of
dyspnea
clear chest x-rays
elevated diaphragms
• TREATMENT: alone or combination of
Glucocorticoids,
Theophylline
Immunosuppressive therapy
Pulmonary manifestations of lupus
PULMONARY HEMORRHAGE
• Rare complication
• High mortality rates (>90%)
• Clinical features:
Dyspnoea
Cough with hemoptysis
Anemia
• CXR: diffuse B/L infiltrates(LZ>UZ)
• BAL: bloody fluid
hemosiderin-laden macrophages
HPE: capillaritis
immune complex deposition
bland hemorrhage
HIGH PROBABILITY: active lupus
high titre of ds DNA antibodies
lupus nephritis
CXR showing B/L infiltrates in the lower
zones
• TREATMENT: one or combination of
high dose steroids
cyclophosphamide
• Plasmapheresis:
severe alveolar haemorrhage refractory
to corticosteroids and cytotoxic agents
Lupus 1997;6:730–3
Cryptogenic organizing pneumonia
(COP)
• BOOP
• Plugs of fibrous tissue in bronchioles and
alveolar ducts
• Dry cough
• CXR: multiple infiltrates
• HRCT: dense consolidation
• Treatment: oral prednisone(1 mg/Kg/day)
Ann Rheum Dis 1991; 50:956
Pulmonary venoocclusive disease
(PVOD)
• Rare cause of PHTN
• Dyspnea and hypoxemia
• Intimal fibrosis---- occlusion of pulmonary
veins
• HRCT CHEST: thickened interlobular septa
lymph node enlargement
ground glass opacities
• Treatment: cyclophosphamide pulse therapy
Vasodilators are contraindicated
Medicine (Baltimore) 2008; 87:220
Pulmonary manifestations of lupus

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Pulmonary manifestations of lupus

  • 2. Introduction SLE( systemic lupus erythematosus) • Autoimmune • Microvascular inflammation • 10:1 female to male ratio • Antibodies directed against double stranded DNA,1 nuclear ribonucleoprotein, Smith (Sm) antigen, Ro/SS-A, and La/SS-B/Ha.
  • 3. Pleuropulmonary manifestations • Pleuritis with or without effusion • Upper and lower airway infections • Acute Lupus Pneumonitis • Chronic interstitial pneumonia • Organizing pneumonia
  • 4. • Pulmonary hypertension • Pulmonary embolism • Respiratory muscle weakness (shrinking lung syndrome) • Alveolar hemorrhage • Mediastinal lymphadenopathy Allergy 2005; 60: 715-734
  • 5. Pleural involvement • Most common manifestation • Asymptomatic • Musculoskeletal pain • Pleuritic chest pain (45-60 %) • Effusions (50-70%) • Dyspnea • Fever
  • 6. • Pleural effusion: B/L exudative, lymphocytic predominant high glucose, low LDH, complement levels LE cells specific • Treatment: Minimal: no treatment, conservative Pleuritic pain: NSAIDs Severe Ds: corticosteroids Chest tube drain/pleurodesis: rarely req
  • 7. CXR showing left sided pleural effusion.
  • 8. CT Chest showing B/L pleural effusion
  • 9. UPPER RESPIRATORY TRACT INFECTION • Cough : mc symptom • Viral • Patients taking corticosteroids or immunosuppresives • Treatment: symptomatic cough syrups, steam inhalation
  • 10. Acute lupus pneumonitis • Non specific • May simulate infections • 1-4 % of patients • Cough, dyspnoea, pleuritic pain, hypoxaemia, and fever • CXR: diffuse acinar infiltrates( u/l as well as b/l) • Sterile sputum/ ET cultures
  • 11. • Histologicaly: alveolar wall damage and necrosis, inflammatory cell infiltration, oedema, haemorrhage,hyaline membrane • CT Scan: alveolitis (a ground glass appearance) fibrosis (a honey comb appearance)
  • 12. • BAL: lymphocytic predominance sterile cultures • Gallium scintigraphy: increased uptake • DLCO : decreased • Video-assisted thoracoscopic biopsy or open lung biopsy: last option
  • 13. Diffuse acinar infiltrates in the right lower zone
  • 14. ARROW showing basal cosolidation with atelectasis
  • 15. Treatment: • Broad spectrum antibiotic cover • Systemic prednisone (1 to 1.5 mg/kg per day in divided doses) • Intravenous pulse glucocorticoids (1 gram of methylprednisolone/ day for 3days) immunosuppressive drugs (cyclophosphamide)
  • 16. Sick patients( tachypnea, hypoxemia) Patients with no response after 72 hours of prednisone • PROGNOSIS: Fulminant course High mortality Poor prog: BAL with eosinophils, postpartum
  • 17. CXR showing right lower zone shadows Improvement of CXR after 4 weeks of therapy
  • 18. INTERSTITIAL LUNG DISEASE • Upto 9 % • Clinical features: Chronic nonproductive cough Dyspnea Decreased exercise tolerance • Diagnosis: pulmonary function tests with exprapulmonary lupus
  • 19. • PFT: restrictive pattern decreased TLC decreased DLCO • HRCT CHEST: ground glass appearance centrilobular nodules thickened bronchovascular bundles and airspaces • LUNG BIOPSY: cellular infiltration with fibrotic changes
  • 20. Patterns include • Nonspecific interstitial pneumonia (NSIP), • Usual interstitial pneumonia(UIP), • Lymphocytic interstitial pneumonia (LIP), • Cryptogenic organizing pneumonia
  • 21. Chronic interstitial pneumonia in a 35 year old woman with SLE.HRCT scan shows extensive ground glass opacities admixed with coarse linear bands and honeycomb cysts.
  • 22. • TREATMENT: depends upon inflammatory or fibrotic pattern • Dual therapy • High glucocorticoids (prednisone 1 to 2 mg/kg/day) and cyclophosphamide • Transition to either azathioprine or mycophenolate mofetil after 6 to 12 months. • Nothing established for fibrotic disease • PIRFENIDONE( tyrosine kinase inhibitor): trial basis
  • 23. PULMONARY HYPERTENSION • Rare complication • 5-10% of patients • Increases with age • 2 % have right heart failure • Dyspnea • Chest pain • Chronic non productive cough
  • 24. • Prominent JVP • Hepatomegaly • Ascites • Peripheral edema • CXR: enlarged pulmonary arteries with clear lung fields. straightening of the left heart border and attenuation of the peripheral vessels
  • 25. CXR showing straightening of the left heart border and attenuation of the peripheral vessels
  • 26. • ECG: RVH • PFT: restrictive pattern decreased DLCO • 2 D ECHO: inc PAP and TR ( tricuspid valve insufficiency) • CT CHEST: dilatation of main pulmonary artery and heterogenesity of lung perfusion
  • 27. • TREATMENT: oxygen anticoagulants vasodilators :bosentan, calcium channel blockers, prostacyclin, endothelial antagonists, sildenafil Intermittent Cyclophosphamide pulse Lupus 2004; 13:105
  • 28. SHRINKING LUNG SYNDROME • Less common manifestation • Dyspnea, • Pleuritic chest pain • Progressive decrease in lung volume • No evidence of interstitial fibrosis or pleural disease on chest CT • myositis or myopathy affecting both diaphragms
  • 29. • DIAGNOSIS: triad of dyspnea clear chest x-rays elevated diaphragms • TREATMENT: alone or combination of Glucocorticoids, Theophylline Immunosuppressive therapy
  • 31. PULMONARY HEMORRHAGE • Rare complication • High mortality rates (>90%) • Clinical features: Dyspnoea Cough with hemoptysis Anemia • CXR: diffuse B/L infiltrates(LZ>UZ)
  • 32. • BAL: bloody fluid hemosiderin-laden macrophages HPE: capillaritis immune complex deposition bland hemorrhage HIGH PROBABILITY: active lupus high titre of ds DNA antibodies lupus nephritis
  • 33. CXR showing B/L infiltrates in the lower zones
  • 34. • TREATMENT: one or combination of high dose steroids cyclophosphamide • Plasmapheresis: severe alveolar haemorrhage refractory to corticosteroids and cytotoxic agents Lupus 1997;6:730–3
  • 35. Cryptogenic organizing pneumonia (COP) • BOOP • Plugs of fibrous tissue in bronchioles and alveolar ducts • Dry cough • CXR: multiple infiltrates • HRCT: dense consolidation • Treatment: oral prednisone(1 mg/Kg/day) Ann Rheum Dis 1991; 50:956
  • 36. Pulmonary venoocclusive disease (PVOD) • Rare cause of PHTN • Dyspnea and hypoxemia • Intimal fibrosis---- occlusion of pulmonary veins • HRCT CHEST: thickened interlobular septa lymph node enlargement ground glass opacities
  • 37. • Treatment: cyclophosphamide pulse therapy Vasodilators are contraindicated Medicine (Baltimore) 2008; 87:220